IMPORTANT SAFETY INFORMATION
WARNING: SEVERE HYPERSENSITIVITY REACTIONS, INFUSION-ASSOCIATED
REACTIONS, and RISK OF ACUTE CARDIORESPIRATORY FAILURE IN SUSCEPTIBLE PATIENTS
Hypersensitivity Reactions
Including Anaphylaxis
Patients treated with NEXVIAZYME have experienced life-threatening
hypersensitivity
reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary
resuscitation equipment, should be readily available during NEXVIAZYME administration. If a severe
hypersensitivity reaction (e.g., anaphylaxis) occurs, NEXVIAZYME should be discontinued immediately
and appropriate medical treatment should be initiated. In patients with severe hypersensitivity
reaction, a desensitization procedure to NEXVIAZYME may be considered.
Infusion-Associated Reactions (IARs)
Patients treated with NEXVIAZYME have experienced severe IARs. If
severe IARs occur, consider immediate discontinuation of NEXVIAZYME, initiation of appropriate
medical treatment, and the benefits and risks of readministering NEXVIAZYME following severe IARs.
Patients with an acute underlying illness at the time of NEXVIAZYME infusion may be at greater risk
for IARs. Patients with advanced Pompe disease may have compromised cardiac and respiratory
function, which may predispose them to a higher risk of severe complications from IARs.
Risk of Acute Cardiorespiratory
Failure in Susceptible Patients
Patients susceptible to fluid volume overload, or those with acute
underlying
respiratory illness or compromised cardiac or respiratory function for whom fluid restriction is
indicated may be at risk of serious exacerbation of their cardiac or respiratory status during
NEXVIAZYME infusion. More frequent monitoring of vitals should be performed during NEXVIAZYME
infusion.
WARNINGS AND PRECAUTIONS
Hypersensitivity Reactions Including Anaphylaxis: Life-threatening hypersensitivity
reactions, including anaphylaxis, have been reported in NEXVIAZYME-treated patients. Some of the
hypersensitivity reactions were IgE mediated. Anaphylaxis signs and symptoms included respiratory
distress, chest discomfort, flushing, cough, erythema, lip swelling, pruritus, swollen tongue,
dysphagia, and rash. Symptoms of severe hypersensitivity reactions included respiratory distress,
erythema, urticaria, tongue edema, and rash. Increased incidence of hypersensitivity reactions was
observed in patients with higher antidrug antibody (ADA) titers. Prior to NEXVIAZYME administration,
consider pretreating with antihistamines, antipyretics, and/or corticosteroids.
Infusion-Associated Reactions: In clinical studies, IARs were reported to occur at any
time during and/or within a few hours after the NEXVIAZYME infusion and were more likely to occur with
higher infusion rates. IARs that led to treatment discontinuation were chest discomfort, cough,
dizziness, erythema, flushing, nausea, ocular hyperemia, and respiratory distress. Increased incidence
of IARs was observed in patients with higher ADA titers. Antihistamines, antipyretics, and/or
corticosteroids can be given prior to NEXVIAZYME administration to reduce the risk of
infusion-associated reactions (IARs). However, IARs may still occur in patients after receiving
pretreatment.
Risk of Acute Cardiorespiratory Failure in Susceptible Patients: Patients susceptible to
fluid volume overload, or those with acute underlying respiratory illness or compromised cardiac or
respiratory function for whom fluid restriction is indicated may be at risk of serious exacerbation of
their cardiac or respiratory status during the NEXVIAZYME infusion. More frequent monitoring of vitals
should be performed during NEXVIAZYME infusion. Some patients may require prolonged observation times.
ADVERSE REACTIONS
The most common adverse reactions (>5%) were headache, fatigue, diarrhea, nausea, arthralgia, dizziness,
myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia and urticaria.
INDICATION
NEXVIAZYME (avalglucosidase alfa-ngpt) is indicated for the treatment of patients 1 year of age and older
with late-onset Pompe disease [lysosomal acid alpha-glucosidase (GAA) deficiency].
Please see full Prescribing Information, including
Boxed WARNING.
